| A Case of Splenectomy in a Patient with Refractory Thrombotic Thrombocytopenic Purpura |
| Jae-Won Lee, M.D.1, Sun-Chul Kim, M.D.1, Se-Won Oh, M.D.1, Jin-Joo Cha, M.D.1, Hye-Won Kim, M.D.1, Chang-Su Boo, M.D.1, Ji-Eun Lee, M.D.2, Young-Joo Kwon, M.D.1 and Heui-Jung Pyo, M.D.1 |
Department of Internal Medicine1
College of Medicine, Korea University, Seoul; Department of Internal Medicine2
Wonkwang University School of Medicine, Gunpo, Korea |
| 증례 : 비장적출술로 호전된 불응성 혈전성 혈소판감소성 자반증 1예 |
| 이재원1, 김선철1, 오세원1, 차진주1, 김혜원1, 부창수1, 이지은2, 권영주1, 표희정1 |
| 고려대학교 의과대학 내과학교실1, 원광대학교 의과대학 내과학교실2 |
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| Abstract |
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The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP) and the survival rate was increased from 10 to 80-90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained dependent on plasma infusions. To discontinue plasma therapy, several attempts using agents such as rituximab, vincristine, and cyclosporine A had been tried, but all failed. After splenectomy, serum LDH and blood platelet count were normalized. Plasmapheresis were we able to discontinue after 2 weeks of splenectomy. Steroid and cyclosporine were tapered off after 3 months and 5 months after splenectomy respectively, and the patient has been staying in remission ever since. We suggest that splenectomy is a worthwhile treatment option in patients with refractory TTP.
Key Words : Thrombotic thrombocytopenic purpura, Splenectomy |
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